The diagnosis and management of intradiaphragmatic extralobar pulmonary sequestrations: a report of 4 cases.

BACKGROUND/PURPOSE: Intradiaphragmatic extralobar pulmonary sequestrations (IDEPSs) are a rare subset of bronchopulmonary sequestrations (BPS). We report the largest series of patients with IDEPS and describe the diagnostic and operative challenges associated with this condition. METHODS: We retrospectively reviewed our experience with fetal and pediatric BPS from 1995 to 2010 to identify patients with IDEPS. RESULTS: We identified 27 patients with BPS and 4 patients in whom the masses were within the diaphragm. In 1 patient, the prenatal ultrasound correctly identified the mass as being within the diaphragm itself, whereas the remaining cases were thought to be intraabdominal or had discordant preoperative imaging findings. The diagnosis of an IDEPS proved challenging to make prospectively using prenatal ultrasound, computed tomography, or magnetic resonance imaging. All patients underwent attempted resection. Two cases required a combined laparoscopic and thoracoscopic approach to accurately localize the mass. The postoperative recovery of these patients was uneventful. CONCLUSIONS: We present the largest reported experience of IDEPS. Because preoperative imaging studies cannot always determine whether a sequestration is intraabdominal, intrathoracic, or intradiaphragmatic, operative planning may pose a challenge. However, the use of minimally invasive approaches can allow exploration of both the thoracic and abdominal cavities with low morbidity.

Anomalous systemic arterial supply to normal basal segments of the right lower lobe in a neonate.

Anomalous systemic arterial supply to normal segments of the lung is a rare congenital anomaly without bronchial abnormalities, which distinguishes this disease from classic bronchopulmonary sequestration. We report on a case presented with congestive heart failure in which a huge systemic arterial blood supply was demonstrated by multidetector computed tomography angiography with three-dimensional reconstruction during the early neonatal period.

Seqüestro pulmonar extralobar: análise anatomopatológica de dois casos em natimortos e revisão da literatura / Extralobar pulmonary sequestration: anatomical analysis of two cases in stillbirths and review of literature

O seqüestro pulmonar é definido como uma massa anormal de tecido pulmonar sem comunicação com a árvore brônquica. É anomalia rara, responsável por 0,15-6,45 por cento das malformações pulmonares congênitas. Quando possui revestimento pleural próprio, chama-se seqüestro pulmonar extralobar (SPE). Este trabalho descreve dois casos de SPE em natimortos (NM) com 32 (1) e 34 (2) semanas de gestação com diagnóstico clínico de hipoxia intra-uterina e adenomatose cística, respectivamente, e faz revisão da literatura. O diagnóstico envolveu análise ultra-sonográfica, sindrômica, macroscópica e microscópica dos NM. Foi observada massa supradiafragmática no hemitórax esquerdo ligada à aorta torácica (1) e ao diafragma (2). As MFs associadas foram agenesia tímica (2), hipoplasia pulmonar (2), pé torto congênito (1) e acondroplasia de membros (2). A microscopia evidenciou, nos dois casos, tecido pulmonar imaturo e pedículo vascularizado e inervado.

Pulmonary sequestration represents an abnormal pulmonary mass that does not communicate with the tracheobronchial tree. It is a rare malformation (MF), accountable for 0.15 percent-6.45 percent of pulmonary congenital MFs. When it has its own pleural covering, it is called extralobar (EBPS). This work describes two cases of EBPS in stillbirths (SB), at 32 (1) and 34 (2) weeks' gestation, with clinical diagnosis of intrauterine hypoxia and cystic adenomatosis, respectively. It also reviews the literature on the subject. The diagnosis involved ultrasonographic, syndromic, macroscopic and microscopic analysis. The macroscopy showed a supradiaphragmatic mass in the left hemithorax linked to thoracic aorta (1) and diaphragm (2). The associated MFs were: thymic agenesis (2), pulmonary hypoplasia (2), clubfoot (1) and achondroplasia (2). Microscopy demonstrated, in both cases, immature pulmonary tissue and vascularized and innervated pedicle.

Neonatal pulmonary sequestration: clinical experience with transumbilical arterial embolization.

Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The exact natural course of PS is not well understood and there are no well-established treatment guidelines for antenatally diagnosed PS. The aim of this study was to describe clinical outcomes in neonates with PS and to evaluate the efficacy of transumbilical arterial embolization (TUE). From 1998 to 2006, total 30 neonatal cases were included. Serial antenatal ultrasound in 26 cases found 6 (23%) regressed lesions, all of which were demonstrated on postnatal chest CT. Six (20%) cases were classified as mixed-type (combined cystic) lesions. Surgery was performed early (during initial hospitalization) in two cases and lately (after the neonatal period) in four cases. TUE was performed for 17 (57%) cases of intrapulmonary PS. Follow-up images obtained a median of 19 months (range, 4-51) after TUE demonstrated complete (9, 53%), partial (5, 29%), and no (3, 18%) regression. The regression rate was significantly higher in solid-type lesions (13/13, 100%) than in mixed-type (1/4, 25%) (P = 0.006). Complications included transient hypertension (two cases, 12%), post-embolization fever (two cases, 12%) and migration of a microcoil (one case, 6%), without long-term morbidities. Natural courses could be observed in 10 cases of extralobar PS and regression was observed in 2 cases (20%) during a median follow-up of 12 months (range, 6-45). A well-designed comparative study is warranted to evaluate the long-term efficacy and safety of TUE.

[Intralobar pulmonary sequestration]. / Intralobäre Lungensequestration.

Sequestration is defined as an area of abnormal pulmonary tissue not connected with the bronchial tree, supplied by an aberrant systemic artery and without a normal pulmonary function. Extralobar (ELS) and intralobar (ILS) forms are distinguished. During the year 2002 the authors diagnosed and operated upon two cases of the intralobar form of pulmonary sequestration, and in last 25 years five cases - 4 x ILS and 1 x ELS. Reported are a 35 year old man with relapsing infections of the sequester and a 21 year old woman where the sequestration was accidentally found without clinical symptoms. The focus was localized in both cases in the left lower lobe of the lungs, anomalous supply arteries derived from the thoracic aorta. Venous drainage of the sequester was different - in the man a systemic drainage via the v. azygos, in the woman via the pulmonary veins was found. In one case the diagnosis was made on the basis of angiography and computer tomography, in the other case it was made on the basis of multidetector CT angiography (MDCTA). Both findings were treated by primary surgical intervention lobectomy. The postoperative course was uneventful.

[Imaging diagnosis for intralobar pulmonary sequestration by subclassification of CT findings in bronchoalveolar structures].

We examined the chest CT findings in 12 cases of intralobar pulmonary sequestration. We classified 4 subtypes by evaluating bronchial and alveolar structures, thus: type A (3 cases), mild cylindrical dilatation of the bronchial structure and hyperlucent alveolar structure; type B (3 cases), marked cylindrical dilatation of the bronchial structure and hyperlucent alveolar structure; type C (2 cases), multicystic dilatation of the bronchial structure and alveolar structure without hyperlucency; and type D (4 cases), multicystic dilatation of the bronchial structure and absence of any alveolar structure. All 77 cases (present and previously reported cases) with CT-documented intralobar pulmonary sequestration could be classified into 4 subtypes: type A 9%, type B 34%, type C 19%, and type D 38%. We concluded that these 4 types were useful for the radiological diagnosis of intralobar pulmonary sequestration.

[Pulmonary sequestration]. / Plauciu sekvestracija.

UNLABELLED: Pulmonary sequestration is rare and serious dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. Pulmonary sequestration masses have autonomic blood supply. For confirmation of the diagnosis we use ultrasound examination, CT scan, MRI, angiopulmography and aortography. AIM OF THE STUDY: To present pathogenesis, diagnostic features and surgical treatment tactics of rare and serious pulmonary dysplasia. MATERIAL AND METHODS: During the period of 1979-2002, we have examined and treated 9 patients because of pulmonary sequestration. We analyzed clinical signs, results of the examination before operation, indications for operation and results of surgical treatment. RESULTS: Seven patients after the operation recovered, 1 patient died due to infection and cardiovascular complications. One patient is supposed to be operated on in the near future. CONCLUSIONS: 1. Pulmonary sequestration is rare and serious bronchial - pulmonary dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. 2. Pulmonary sequestrations are classified as intralobar, extralobar and extrapulmonary sequestration. 3. Pulmonary sequestration is often associated with cardiovascular dysplasias and diaphragmatic malformations. 4. Methods of examination: X-ray examination, CT scan, MRI, ultrasound examination, aortography. 5. Complications of pulmonary sequestration: pneumonia, pleuritis, necrosis of the sequestration, chylothorax, respiratory distress syndrome, hemothorax, cystic transformation, and pneumothorax. 6. Treatment - surgery or embolisation of abnormal supply vessels.

Revisit on congenital bronchopulmonary vascular malformations: a haphazard branching theory of malinosculations and its clinical classification and implication.

We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.

The multiple facets of pulmonary sequestration.

PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. METHODS: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.

[Intralobar sequestration of the lung in an adult (type 1 of pryce). Treatment by coil embolization]. / Séquestration pulmonaire intralobaire de type pryce 1 chez l'adulte. Vaso-occlusion par spires métalliques.

A 34 year old patient presented with recurrent hemoptysis. A chest radiograph was normal. Helical CT angiogram of the chest with tridimensional reconstructions demonstrated an intralobar pulmonary vascular sequestration Pryce I-type of the right lower lobe. The angiography confirmed the diagnosis showing a large feeding artery arising from the coeliac trunk. An embolization of the feeding artery using metallic coils was performed. Transient pulmonary infarction occurred immediately after treatment. At two years follow-up, the patient remains asymptomatic. Percutaneous embolization of this rare pulmonary vascular malformation is an alternative to surgical treatment.

[A case of Pryce type I intrapulmonary sequestration].

A twenty-year-old asymptomatic man hospitalized because of a vascular murmur and abnormal shadow in the left lower lung on X-ray film. An aortogram revealed an abnormal artery arising from the descending thoracic aorta and supplying the left basal segment, which had no other pulmonary arteries. Although lung ventilation scintigraphy demonstrated reduced ventilation to the left lower lobe, bronchogram showed an almost normal bronchial tree except that peripheral branches were slightly thin. A clinical diagnosis of Pryce type I intrapulmonary sequestration was made, and left lower lobectomy was performed successfully. We have analyzed 31 cases of Pryce type I intrapulmonary sequestration in Japan. A vascular murmur is often heard, and a chest X-ray usually shows either a mass shadow or increased vascular markings. In most of those cases, an abnormal artery arises from the descending thoracic aorta and it supplies the left basal segment. Because this type of sequestration causes hemoptysis and infections, surgical intervention is indicated.

[Pryce type I intralobar pulmonary sequestration].

A 53-year-old woman had a abnormal shadow in the left lower lung field on a chest radiograph, and occasional bloody sputum. CT scans and an MRI showed an abnormal vessel shadow between the aorta and the heart. An aortogram showed an abnormal artery originating from the descending aorta and entering the left lower lobe, and a pulmonary angiogram revealed no blood supply to the left basal segments. Pryce type I pulmonary sequestration was diagnosed. The patient underwent surgery, and the abnormal artery, 13 mm in diameter, was found to be connected from the descending aorta to the left lower lobe. The abnormal artery was dissected, and the left lower lobe was removed. The postoperative course was uneventful. We collected data on 24 cases of Pryce type I pulmonary sequestration reported in Japan. Such cases are comparatively rare and males are affected more often than females. The patient in the present case was the oldest woman to have undergone surgery for this condition in Japan. In patients who underwent surgery the greatest diameter of the abnormal artery was 18 mm.

Secuestro pulmonar

En un período de 20 años, se operaron 15 casos de secuestro pulmonar (SP); 11 fueron hombres, y la edad media fue de 34 años (rango 10-56 años). Doce pacientes fueron sintomáticos. La angiografía preoperatoria practicada a trece pacientes, fue diagnóstica en todos los casos. En 86 por ciento de los pacientes el SP se localizó en el segmento basal posterior. La cirugía fue practicada en todos los casos sin mortalidad; catorce fueron intralobares (SIL) y no fue estralobar (SEL). En un paciente se encontró incidentalmente un carcinoma bronquioloalveolar. El SP aunque infrecuente es importante de tener en mente ya que puede asociarse a infecciones pulmonares crónicas severas y otras enfermedades muy incapacitantes o potencialmente letales, tales como el cáncer de pulmón. La cirugía es el tratamiento de elección, e idealmente requiere que el diagnóstico se haga preoperatoriamente, para detectar el vaso anómalo y evitar accidentes quirúrgicos que puede ser fatales; esto es ahora más fácil con la nueva tecnología no invasiva en imágenes, sin embargo un alto índice de sospecha es necesario.